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Angiographic Findings of Persistent Primitive Hepatic Venous Plexus with Underdevelopment of the Infrahepatic Inferior Vena Cava in Pediatric Patients

¹ Department of Diagnostic Imaging, Hospital for Sick Children and University of Toronto, 555 University Ave., Toronto M5G 1X8, Canada.
² Division of Cardiology, Hospital for Sick Children and University of Toronto, Toronto M5G 1X8, Canada.
³ Department of Critical Care Medicine, Hospital for Sick Children and University of Toronto, Toronto M5G 1X8, Canada.
⁴ Department of Pediatrics, Hospital for Sick Children and University of Toronto, Toronto M5G 1X8, Canada.

View larger version (90K): [in a new window]   Fig. 1. —15-year-old girl with group 1 anomaly and scimitar syndrome, dextrocardia, anomalous right pulmonary venous drainage to inferior vena cava (IVC), right pulmonary artery hypoplasia, and azygos continuation of IVC. This inferior venacavogram was obtained after surgery to patch IVC and to reroute anomalous right pulmonary veins and shows persistent hepatic venous plexus (asterisk). RA = right atrium, HV = hepatic vein.

View larger version (104K): [in a new window]   Fig. 2. —2-year-old girl with group 1 anomaly and scimitar syndrome, dextrocardia, anomalous right pulmonary venous drainage to inferior vena cava (IVC), and right pulmonary artery hypoplasia without azygos continuation of the IVC. This inferior venacavogram shows persistent hepatic venous plexus is shown (asterisk). RA = right atrium, HV = hepatic vein.

View larger version (96K): [in a new window]   Fig. 3. —2-year-old girl with group 1 anomaly and congenital pulmonary vein stenosis, ventricular septal defect, and bilateral superior vena cavae. She underwent ventricular septal defectclosure and repair of pulmonary vein stenosis. Inferior venacavogram that shows persistent hepatic venous plexus (asterisk) was performed to investigate abnormal catheter course during catheterization to assess recurrent pulmonary vein stenosis. RA = right atrium, HV = hepatic vein, IVC = inferior vena cava.

View larger version (119K): [in a new window]   Fig. 4. —7-year-old boy with group 2 anomaly and left atrial isomerism, double outlet right ventricle, unbalanced atrioventricular canal defect (right dominant), coarctation of aorta, bilateral superior vena cavae, and azygos continuation of inferior vena cava. Previous surgeries (not shown) included Damus-Kaye-Stansel anastomosis [13], coarctation repair, right modified Blalock-Taussig shunt, Kawashima procedure [7], and bidirectional left superior vena cava to pulmonary artery anastomosis. Left renal venogram shows renal-portal-hepatic venous plexus (asterisk). PV = pulmonary vein, LRV = left renal vein.

View larger version (112K): [in a new window]   Fig. 5A. —4-year-old girl with group 2 anomaly and left atrial isomerism, unbalanced atrioventricular canal defect (right dominant), pulmonary and left atrioventricular valve atresia, transposed great arteries, and azygos continuation of inferior vena cava. Previous surgeries (not shown) included right modified Blalock-Taussig shunt, atrial septectomy and resection of left atrial membrane, left pulmonary arterioplasty, and Kawashima procedure [7] (B). Inferior venacavogram shows renal-portal-hepatic venous plexus (asterisk). RA = right atrium, HV = hepatic vein, RRV = right renal vein, IVC = inferior vena cava.

View larger version (148K): [in a new window]   Fig. 5B. —4-year-old girl with group 2 anomaly and left atrial isomerism, unbalanced atrioventricular canal defect (right dominant), pulmonary and left atrioventricular valve atresia, transposed great arteries, and azygos continuation of inferior vena cava. Previous surgeries (not shown) included right modified Blalock-Taussig shunt, atrial septectomy and resection of left atrial membrane, left pulmonary arterioplasty, and Kawashima procedure [7] (B). Superior venacavogram shows Kawashima procedure or anastomosis of azygos (Az) and superior vena cava (SVC) with pulmonary artery (PA). Note decompression down azygos (arrow) to right atrium via renal-portal-hepatic venous plexus (See asterisk, Fig. 5A), causing persistent and unexpected arterial desaturation after surgery.

View larger version (114K): [in a new window]   Fig. 6. —18-month-old girl with group 2 anomaly and left atrial isomerism, double-outlet right ventricle, rightward and anterior aorta, unbalanced atrioventricular canal defect (right dominant), pulmonary stenosis, bilateral superior vena cavae, and interrupted inferior vena cava (IVC) with azygos continuation. Previous surgeries (not shown) included right modified Blalock-Taussig shunt and Kawashima procedure [7] with ligation of left superior vena cava. Note renal-portal-hepatic venous plexus (asterisk). RA = right atrium, PV = pulmonary vein, RRV = right renal vein.

View larger version (130K): [in a new window]   Fig. 7. —18-month-old girl with group 2 anomaly and left atrial isomerism, double outlet right ventricle, rightward and anterior aorta, unbalanced atrioventricular canal defect (right dominant), and interrupted inferior vena cava (IVC) with azygos continuation. Previous surgeries (not shown) included pulmonary artery banding. Low inferior venacavogram obtained before Kawashima procedure [7] shows persistence of renal-portal-hepatic venous plexus (asterisk). PV = pulmonary vein, LRV = left renal vein.

View larger version (92K): [in a new window]   Fig. 8A. —4-year-old boy with left atrial isomerism, double outlet right ventricle, rightward and anterior aorta, atrioventricular canal defect, bilateral superior vena cavae, hemiazygos continuation of inferior vena cava (IVC) to left superior vena cava, and hypoplastic hepatic IVC. Previous interventions (not shown) included pulmonary artery banding, pulmonary artery angioplasty, and Kawashima procedure [7] with occlusion of right superior vena cava. Inferior venacavogram shows hemiazygos (HA) continuation of IVC with persistence of patient, albeit underdeveloped, IVC. Patent IVC allows decompression of systemic venous return away from pulmonary artery to atrium, leading to hypoxemia.

View larger version (86K): [in a new window]   Fig. 8B. —4-year-old boy with left atrial isomerism, double outlet right ventricle, rightward and anterior aorta, atrioventricular canal defect, bilateral superior vena cavae, hemiazygos continuation of inferior vena cava (IVC) to left superior vena cava, and hypoplastic hepatic IVC. Previous interventions (not shown) included pulmonary artery banding, pulmonary artery angioplasty, and Kawashima procedure [7] with occlusion of right superior vena cava. Balloon occlusion inferior venacavogram of underdeveloped hepatic IVC is required to show persistence of renal-portal-hepatic venous plexus (asterisk), which provides another pathway to decompress the pulmonary circulation. Thus, coil embolization of persistent hepatic IVC alone would not eliminate right-to-left shunt down hemiazygos to atrium. HA = hemiazygos continuation of IVC.

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